Transl Med UniSa. 2011 Oct 17;1:195-212. Print 2011 Sep.
Translational medicine @ UniSa
Giorgina Mieli-Vergani, Diego Vergani
PMID: 23905033 PMCID: PMC3728841
Autoimmune liver disorders in childhood include autoimmune hepatitis and autoimmune sclerosing cholangitis. These inflammatory liver disorders are characterised histologically by interface hepatitis, biochemically by elevated transaminase levels and serologically by autoantibodies and increased levels of immunoglobulin G. Autoimmune hepatitis is particularly aggressive in children and progresses rapidly unless immunosuppressive treatment is started promptly. With appropriate treatment 80% of patients achieve remission and long-term survival. Autoimmune sclerosing cholangitis responds to the same treatment used for autoimmune hepatitis in regards to parenchymal inflammation, but bile duct disease progresses in about 50% of cases, leading to a worse prognosis and higher transplantation requirement; it has a high recurrence rate post liver transplant. New strategies aiming at treating unresponsive patients and at curbing specifically the liver autoimmune attack, without provoking unwanted systemic side effects, are being investigated and may be available within the next 5 years.
Keywords: autoimmune hepatitis; autoimmune sclerosing cholangitis; azathioprine; calcineurin inhibitors; corticosteroids; mycophenolate mofetil; regulatory T-cells; ursodeoxycholic acid
